DermatoFibrosarcoma Protuberans - A rare dermatologic malignancy: 120 case reports

Background: The dermatofibrosarcoma protuberans is a rare skin tumor with a low grade malignancy. It is characterized by a random and asymmetric local extension and a spontaneous tendency to local recurrence. This characteristic is the main difficulty in the management of dermatofibrosarcoma protuberans. The aim of our study was to evaluate our experience in the management of this disease and to clarify the epidemiological, clinical, therapeutic and prognostic characteristics of this rare tumor. Methods: This was a retrospective, single-center, covering 120 cases of dermatofibrosarcoma protuberans treated at the Institute Salah Azaiez, over a period of 21 years from 1 January 1992 to 31 December 2012. Results: The average age at diagnosis was 41.5 years, ranging from 7 to 82 years. . Our population was consisted of 51 women and 69 men. The chief complaint most frequently found was the rapid growth of a nodule (39.2% of cases). The average consultation time was 51 months. The predilection was the trunk in 71 patients (59%) and members in 42 patients (35%). The average lesion size was 6 cm, with a range of 1 to 20 cm. The most common appearance was an isolated nodule in 60% of cases. Ultrasound soft tissue was done in 15 cases (12.5%). Therapeutically, our patients underwent a wide excision in 118 cases and adjuvant radiotherapy in two cases. We observed nine cases of recurrence after a median follow up of 29 months. We observed ten cases of sarcomatous transformation six of which have developed lethal metastases. Conclusion: The prognosis of this disease depends on the surgery performed in first intention which must be complete from the start. Any inappropriate surgery increases the risk of recurrence, sarcomatous degeneration and metastasis.